Septic shock after liver transplantation for Caroli's disease: clinical improvement after treatment with C1-esterase inhibitor

The extent of complement and contact activation is related to outcome in se psis. A low functional index of their main blocker C1-esterase inhibitor (C 1-INH) is considered as a relative deficiency of C1-INH and might contribut e to the development of fatal complications in the intensive care unit. The first results of therapeutic intervention with C1-INH concentrate in septi c shock are promising. We report on our experience of C1-INH concentrate ad ministration in a young woman with Caroli's disease as ultimate rescue ther apy for septic shock with capillary leakage syndrome after combined liver a nd kidney transplantation. No focus of infection was detectable and thus su rgical intervention was not indicated. Antibiotic therapy at that time incl uded vancomycin, tobramycin, meropenem and fluconazol. Hemodynamic stabiliz ation occurred within hours after administration of C1-INH concentrate. Sim ultaneously a reduction in vasopressor medication was possible and negative fluid balance was achieved.